Abdominal pain, jaundice and abdominal masses are the 3 typical symptoms of the disease, and infants are most often seen for abdominal masses or jaundice. The abdominal pain is mostly confined to the upper abdomen, right upper abdomen or around the umbilicus. The nature and degree of abdominal pain are variable; it may be pseudo-biliary colic or intermittent abdominal pain, but most of them are distending or pulling pains that are not significantly related to eating. Persistent severe pain with fever is often a manifestation of cholangitis and is accompanied by gastrointestinal symptoms such as nausea, vomiting and anorexia. Jaundice is intermittent and characterized by intervals of varying length, with an occurrence rate of 51-70%. Severe jaundice may be accompanied by itching of the skin and general discomfort. The appearance and deepening of jaundice may indicate distal common bile duct stenosis or coexistence of biliary tract infection. When bile is drained smoothly, jaundice may decrease or subside. The abdominal mass is often the first sign of a sick child and is present in 90-100% of cases. The mass is located in the right upper abdomen, below the hepatic margin, and the upper border is mostly covered by the hepatic margin. In large cases, it may extend beyond the midline of the abdomen or reach below the umbilicus. The surface of the mass is smooth, spherically cystic, and well-defined. In some cases, the cyst is more tense or can move mildly. In addition, there is fever, which can be as high as 38-39°C, due to bacterial intracapsular inflammation or intrahepatic bile duct infection. Nausea and vomiting can be caused by gastrointestinal reactions of the sick child or by the cyst compressing the intestinal canal. Repeated hyperthermia and vomiting can lead to failure of the sick child.